Scheibel E, Feddersen C, Hertz H
Scand J Haematol. 1985 May;34(5):378-84. doi: 10.1111/j.1600-0609.1985.tb00765.x.
3 patients with haemophilia A and inhibitor against Factor VIII were developing progressive haemophiliac arthropathy due to the non-feasibility of prophylactic treatment. In order to suppress inhibitor formation, long-term treatment with high-dose Factor VIII (100 units per kg body weight twice daily) was initiated. Prothrombin complex concentrate was given only for bleeding episodes. Though all 3 patients were high responders, they presented different treatment courses. 2 became low responders after 4 and 11 months' treatment, respectively. 1 patient had no demonstrable inhibitor after start of treatment. In all 3 patients, prophylactic treatment was established, in 1 case still with increased doses compared to non-inhibitor patients. The high-dose Factor VIII treatment makes in possible to provide prophylactic treatment for the high-responder inhibitor patients. However, the extremely high costs represent a serious obstacle to this treatment.
3例甲型血友病且对凝血因子VIII产生抑制物的患者，由于预防性治疗不可行，正在发展为进行性血友病性关节病。为抑制抑制物形成，开始采用高剂量凝血因子VIII（每公斤体重100单位，每日两次）进行长期治疗。仅在出血发作时给予凝血酶原复合物浓缩剂。尽管所有3例患者都是高反应者，但他们呈现出不同的治疗过程。2例患者分别在治疗4个月和11个月后成为低反应者。1例患者在开始治疗后未检测到抑制物。在所有3例患者中，均确立了预防性治疗，其中1例与无抑制物的患者相比，剂量仍有所增加。高剂量凝血因子VIII治疗使得为高反应性抑制物患者提供预防性治疗成为可能。然而，极高的费用是这种治疗的严重障碍。
